Prion Diseases - PowerPoint PPT Presentation

Prion Diseases. Microbes and Society Fall 2007. What is a Prion?. Prion- small proteinaceous infectious particles which resist inactivation by procedures that modify nucleic acids

Copyright Complaint Adult Content Flag as Inappropriate

I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.

Download Presentation

PowerPoint Slideshow about 'Prion Diseases' - julio

An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.

TSE’s in Animals disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response. (CDC)

Scapie- sheep, first known TSE been around for 100’s of years. Sheep get it by ingestion off of pasture or transplacental transmission

Mad Cow Disease or BSE- cows. First cases may have occurred by feeding scrapie infected tissue to cows

Chronic Wasting Disease (CWD)- deer, elk

Transmissible Mink Encephalopathies (TME)- mink

Animal TSE’s and Society disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response. (CDC)

BSE in Great Britain cost millions of dollars, loss of life, a crash in the beef industry and fostered mistrust of the government. Others?

More TSE’s and Animals disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response. (CDC)

Scrapie-

Packers and producers have had difficulty finding options for disposal of sheep offal and dead sheep causing packers and producers to incur significant increases in disposal costs.

• Other countries have expressed concerns and have indicated that they may prohibit or restrict certain ruminant products because the United States has scrapie.

• Domestic and international markets for U.S. sheep–derived meat and bone meal have been adversely affected.

The combination of all of these factors has led to the decision to develop a strong scrapie eradication program in the United States

TSE’s and Humans disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response. (CDC)

Creutzfeldt-Jakob Disease (CJD)

Variant Creutzfeldt-Jakob Disease (vCJD)

Gerstmann-Straussler-Scheinker Syndrome

Fatal Familial Insomnia

Kuru

Classic CJD disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response. (CDC)

Classic CJD has been recognized since the early 1920s. The most common form of classic CJD is believed to occur sporadically, caused by the spontaneous transformation of normal prion proteins into abnormal prions. This sporadic disease occurs worldwide, including the United States, at a rate of approximately one case per 1 million population per year, although rates of up to two cases per million are not unusual.

vCJD disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response. (CDC)

There is now strong scientific evidence that the agent responsible for the outbreak of prion disease in cows, BSE, is the same agent responsible for the outbreak of vCJD in humans. Both disorders are invariably fatal brain diseases with unusually long incubation periods measured in years, and are caused by an unconventional transmissible agent.

However, risk of acquiring vCJD through infected cow meat is still low. Why?

Comparison of classic CJD and vCJD disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response. (CDC)

The median age at death for vCJD patients is 28 years, compared with 68 years for patients with classic CJD.

The median duration of illness for vCJD is 14 months, compared to 5 months for classic CJD.

Kuru disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response. (CDC)

Kuru is a rare and fatal brain disorder that occurred at epidemic levels during the 1950s-60s among the Fore people in the highlands of New Guinea.

The disease was the result of the practice of ritualistic cannibalism among the Fore, in which relatives prepared and consumed the tissues (including brain) of deceased family members.

Brain tissue from individuals with kuru was highly infectious, and the disease was transmitted either through eating or by contact with open sores or wounds.

Inherited Human TSE’s disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response. (CDC)

Gerstmann-Straussler-Scheinker Syndrome- a rare inherited dementia that presents in the later years.

Treatment disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response. (CDC)

Nothing yet, but immune cells may be what carries the protein to the lymph nodes and eventually the brain.

Prevention- food animals have strict diets which limit or prevent animals from ingesting CNS material that may contain prions.